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Published in: Journal of Gastrointestinal Cancer 4/2011

01-12-2011 | Case Report

Non-Functioning Pancreatic Neuroendocrine Tumors—A Case Report and Review of Literature

Authors: Murtuza M. Rampurwala, Anupam Kumar, Subramanian Kannan, Pamela Kowalczyk, Surendra Khera

Published in: Journal of Gastrointestinal Cancer | Issue 4/2011

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Abstract

Purpose

Neuroendocrine tumors of pancreas (PNET) are rare pancreatic neoplasms comprising 1–2% of all pancreatic tumors. The overall prognosis and long-term survival for PNET patients is far better than for patients with exocrine pancreatic cancer. PNETs are classified as functional or nonfunctional based on the presence or absence of a specific clinical syndrome associated with hormone oversecretion.

Methods

We present the case of a 36-year-old female with epigastric and right upper quadrant abdominal pain for 3 months associated with decreased appetite, early satiety and a 20-lb weight loss. On examination, she was cachectic with hepatomegaly.

Results

Laboratory assays showed elevated liver and pancreatic enzymes. On computed tomography (CT) scan of the abdomen and pelvis, there was a low-attenuation mass in the distal pancreatic tail measuring 4.7 × 2.4 cm with multiple liver masses, omental implants, left ovarian mass, and a small amount of ascites. CT-guided liver biopsy on pathology was consistent with a well-differentiated pancreatic neuroendocrine carcinoma with metastasis to the liver. Assays for biomarkers of pancreatic neuroendocrine tumors showed an elevated chromogranin A with normal to non-specific elevations of the rest.

Conclusions

The patient and her family declined palliative chemoembolization of the liver lesions or palliative chemotherapy and desired home hospice. We describe here the presentation and course of the case as well as a literature review of PNET with particular emphasis on nonfunctioning PNETs.
Literature
1.
go back to reference Modlin IM, Oberg K, Chung DC, et al. Gastroenteropancreatic neuroendocrine tumors. Lancet Oncol. 2008;9:61–72.PubMedCrossRef Modlin IM, Oberg K, Chung DC, et al. Gastroenteropancreatic neuroendocrine tumors. Lancet Oncol. 2008;9:61–72.PubMedCrossRef
2.
go back to reference Kang C, Park S, Kim K, et al. Surgical experiences of functioning neuroendocrine neoplasm of pancreas. Yonsei Med J. 2006;47:833–9.PubMedCrossRef Kang C, Park S, Kim K, et al. Surgical experiences of functioning neuroendocrine neoplasm of pancreas. Yonsei Med J. 2006;47:833–9.PubMedCrossRef
4.
go back to reference Massironi S, Sciola V, Peracchi M, et al. Neuroendocrine tumors of the gastro-entero-pancreatic system. World J Gastroenterol. 2008;14(35):5377–84.PubMedCrossRef Massironi S, Sciola V, Peracchi M, et al. Neuroendocrine tumors of the gastro-entero-pancreatic system. World J Gastroenterol. 2008;14(35):5377–84.PubMedCrossRef
5.
go back to reference Oberg K, Eriksson B. Endocrine tumors of pancreas. Best Pract Clin Gastroenterol. 2005;19:753–81.CrossRef Oberg K, Eriksson B. Endocrine tumors of pancreas. Best Pract Clin Gastroenterol. 2005;19:753–81.CrossRef
6.
go back to reference Ramage JK, Davies AH, Ardill J, et al. Guidelines for the management of gastroenteropancreatic neuroendocrine (including carcinoid) tumors. Gut. 2005;54 Suppl 4:iv1–16.PubMedCrossRef Ramage JK, Davies AH, Ardill J, et al. Guidelines for the management of gastroenteropancreatic neuroendocrine (including carcinoid) tumors. Gut. 2005;54 Suppl 4:iv1–16.PubMedCrossRef
7.
go back to reference Kimura W, Kuroda A, Morioka Y. Clinical pathology of endocrine tumors of the pancreas. Analysis of autopsy cases. Dig Dis Sci. 1991;36:933–42.PubMedCrossRef Kimura W, Kuroda A, Morioka Y. Clinical pathology of endocrine tumors of the pancreas. Analysis of autopsy cases. Dig Dis Sci. 1991;36:933–42.PubMedCrossRef
8.
go back to reference Ehehalt F, Saeger H, Schmidt C, et al. Neuroendocrine tumors of the pancreas. Oncologist. 2009;14:456–67.PubMedCrossRef Ehehalt F, Saeger H, Schmidt C, et al. Neuroendocrine tumors of the pancreas. Oncologist. 2009;14:456–67.PubMedCrossRef
9.
10.
go back to reference Ong SL, Garcea G, Pollard CA, et al. A fuller understanding of pancreatic neuroendocrine tumors combined with aggressive management improves outcome. Pancreatology. 2009;9:583–600.PubMedCrossRef Ong SL, Garcea G, Pollard CA, et al. A fuller understanding of pancreatic neuroendocrine tumors combined with aggressive management improves outcome. Pancreatology. 2009;9:583–600.PubMedCrossRef
11.
go back to reference Anderson M, Carpenter S, Thompson N, et al. Endoscopic ultrasound is highly accurate and directs management in patients with neuroendocrine tumors of pancreas. Am J Gastroenterol. 2000;95:2271–7.PubMedCrossRef Anderson M, Carpenter S, Thompson N, et al. Endoscopic ultrasound is highly accurate and directs management in patients with neuroendocrine tumors of pancreas. Am J Gastroenterol. 2000;95:2271–7.PubMedCrossRef
12.
go back to reference Peracchi M, Conte D, Gebbia C, et al. Plasma chromogranin A in patients with sporadic gastro-entero-pancreatic neuroendocrine tumors or multiple endocrine neoplasia type 1. Eur J Endocrinol. 2003;148:39–43.PubMedCrossRef Peracchi M, Conte D, Gebbia C, et al. Plasma chromogranin A in patients with sporadic gastro-entero-pancreatic neuroendocrine tumors or multiple endocrine neoplasia type 1. Eur J Endocrinol. 2003;148:39–43.PubMedCrossRef
13.
go back to reference Fesinmeyer MD, Austin MA, Li CI, et al. Differences in survival by histologic type of pancreatic cancer. Cancer Epidemiol Biomark Prev. 2005;14:1766–73.CrossRef Fesinmeyer MD, Austin MA, Li CI, et al. Differences in survival by histologic type of pancreatic cancer. Cancer Epidemiol Biomark Prev. 2005;14:1766–73.CrossRef
14.
go back to reference Heitz PU, Komminoth P, Perrin A, et al. Pancreatic endocrine tumors: introduction. In: DeLellis DA, Lloyd RV, Heitz PU, et al., editors. Pathology and genetics of tumors of endocrine organs; WHO Classification of Tumors. Lyon: IARC Press; 2004. p. 177–82. Heitz PU, Komminoth P, Perrin A, et al. Pancreatic endocrine tumors: introduction. In: DeLellis DA, Lloyd RV, Heitz PU, et al., editors. Pathology and genetics of tumors of endocrine organs; WHO Classification of Tumors. Lyon: IARC Press; 2004. p. 177–82.
15.
go back to reference Solcia E, Klöppel G, Sobin LH. Histological typing of endocrine tumors. In: WHO international histological classification of tumors. 2nd ed. Berlin: Springer; 2000. p. 56–70 Solcia E, Klöppel G, Sobin LH. Histological typing of endocrine tumors. In: WHO international histological classification of tumors. 2nd ed. Berlin: Springer; 2000. p. 56–70
16.
go back to reference Rindi G, Kloppel G, Alhman H, et al. TNM staging of foregut (neuro) endocrine tumors: a consensus proposal including a grading system. Virchows Arch. 2006;449:395–401.PubMedCrossRef Rindi G, Kloppel G, Alhman H, et al. TNM staging of foregut (neuro) endocrine tumors: a consensus proposal including a grading system. Virchows Arch. 2006;449:395–401.PubMedCrossRef
17.
go back to reference Rindi G, Kloppel G, Couvelard A, et al. TNM staging of midgut and hindgut (neuro) endocrine tumors: a consensus proposal including a grading system. Virchows Arch. 2007;451:757–62.PubMedCrossRef Rindi G, Kloppel G, Couvelard A, et al. TNM staging of midgut and hindgut (neuro) endocrine tumors: a consensus proposal including a grading system. Virchows Arch. 2007;451:757–62.PubMedCrossRef
18.
go back to reference Shan Y, Sy E, Lin P. Role of somatostatin in the prevention of pancreatic stump-related morbidity following elective pancreato-duodenectomy in high-risk patients and elimination of surgeon-related factors: prospective, randomized, controlled trial. World J Surg. 2003;27:709–14.PubMedCrossRef Shan Y, Sy E, Lin P. Role of somatostatin in the prevention of pancreatic stump-related morbidity following elective pancreato-duodenectomy in high-risk patients and elimination of surgeon-related factors: prospective, randomized, controlled trial. World J Surg. 2003;27:709–14.PubMedCrossRef
19.
go back to reference Ben-Shlomo A, Melmed S. Pasireotide—a somatostatin analog for the potential treatment of acromegaly, neuroendocrine tumors and Cushing’s disease. IDrugs. 2007;10:885–95.PubMed Ben-Shlomo A, Melmed S. Pasireotide—a somatostatin analog for the potential treatment of acromegaly, neuroendocrine tumors and Cushing’s disease. IDrugs. 2007;10:885–95.PubMed
20.
go back to reference Hubina E, Nanzer A, Hanson M, et al. Somatostatin analogues stimulate p27 expression and inhibit the MAP kinase pathway in pituitary tumors. Eur J Endocrinol. 2006;155:371–9.PubMedCrossRef Hubina E, Nanzer A, Hanson M, et al. Somatostatin analogues stimulate p27 expression and inhibit the MAP kinase pathway in pituitary tumors. Eur J Endocrinol. 2006;155:371–9.PubMedCrossRef
21.
go back to reference Dimou AT, Syrigos KN, Saif MW. Neuroendocrine tumors of the pancreas: what’s new? Highlights from the “2010 ASCO Gastrointestinal Cancers Symposium”. Orlando, FL, USA. 22–24 January 2010. JOP. 2010;11(2):135–8.PubMed Dimou AT, Syrigos KN, Saif MW. Neuroendocrine tumors of the pancreas: what’s new? Highlights from the “2010 ASCO Gastrointestinal Cancers Symposium”. Orlando, FL, USA. 22–24 January 2010. JOP. 2010;11(2):135–8.PubMed
22.
go back to reference Arnold R, Rinke A, Schmidt C, et al. Endocrine tumors of gastrointestinal tract: chemotherapy. Best Pract Res Clin Gastroenterol. 2005;19(4):649–56.PubMedCrossRef Arnold R, Rinke A, Schmidt C, et al. Endocrine tumors of gastrointestinal tract: chemotherapy. Best Pract Res Clin Gastroenterol. 2005;19(4):649–56.PubMedCrossRef
23.
go back to reference Ramanathan R, Cnaan A, Hahn R, et al. Phase II trial of dacarbazine in advanced pancreatic islet cell carcinoma. Study of the Eastern Cooperative Oncology Group E6282. Ann Oncol. 2001;12:1139–43.PubMedCrossRef Ramanathan R, Cnaan A, Hahn R, et al. Phase II trial of dacarbazine in advanced pancreatic islet cell carcinoma. Study of the Eastern Cooperative Oncology Group E6282. Ann Oncol. 2001;12:1139–43.PubMedCrossRef
24.
go back to reference Oberg K. Interferon in the management of neuroendocrine GEP tumors. Digestion. 2000;62(suppl I):92–7.PubMed Oberg K. Interferon in the management of neuroendocrine GEP tumors. Digestion. 2000;62(suppl I):92–7.PubMed
25.
go back to reference Dirix LY, Vermeulen PB, Fierens H, et al. Long term results of continuous treatment with recombinant interferon alpha in patients with metastatic carcinoid tumors—an anti-angiogenic effect? Anticancer Drugs. 1996;7:175–81.PubMedCrossRef Dirix LY, Vermeulen PB, Fierens H, et al. Long term results of continuous treatment with recombinant interferon alpha in patients with metastatic carcinoid tumors—an anti-angiogenic effect? Anticancer Drugs. 1996;7:175–81.PubMedCrossRef
26.
go back to reference Yao JC. Neuroendocrine tumors. Molecular targeted therapy for carcinoid and islet-cell carcinoma. Best Pract Res Clin Endocrinol Metab. 2007;21:163–72.PubMedCrossRef Yao JC. Neuroendocrine tumors. Molecular targeted therapy for carcinoid and islet-cell carcinoma. Best Pract Res Clin Endocrinol Metab. 2007;21:163–72.PubMedCrossRef
27.
go back to reference Yao JC, Hoff PM. Molecular targeted therapy for neuroendocrine tumors. Hematol Oncol Clin North Am. 2007;21:575–81.PubMedCrossRef Yao JC, Hoff PM. Molecular targeted therapy for neuroendocrine tumors. Hematol Oncol Clin North Am. 2007;21:575–81.PubMedCrossRef
28.
go back to reference Forrer F, Valkema R, Kwekkeboom DJ, et al. Neuroendocrine tumors. Peptide receptor radionuclide therapy. Best Pract Res Clin Endocrinol Metab. 2007;21:111–29.PubMedCrossRef Forrer F, Valkema R, Kwekkeboom DJ, et al. Neuroendocrine tumors. Peptide receptor radionuclide therapy. Best Pract Res Clin Endocrinol Metab. 2007;21:111–29.PubMedCrossRef
29.
go back to reference Kwekkeboom DJ, Bakker WH, Kam BL, et al. Treatment of patients with gastro-entero-pancreatic (GEP) tumors with the novel radiolabelled somatostatin analogue [177Lu- DOTA (0), Tyr3] octreotate. Eur J Nucl Med Mol Imaging. 2003;30:417–22.PubMedCrossRef Kwekkeboom DJ, Bakker WH, Kam BL, et al. Treatment of patients with gastro-entero-pancreatic (GEP) tumors with the novel radiolabelled somatostatin analogue [177Lu- DOTA (0), Tyr3] octreotate. Eur J Nucl Med Mol Imaging. 2003;30:417–22.PubMedCrossRef
30.
go back to reference Gulec S, Mesloras G, Dezarn W et al. Safety and efficacy of Y-90 microsphere treatment in patients with primary and metastatic liver cancer: tumor selectivity of the treatment as a function of tumor to liver flow ratio. J Transl Med. 2007;5–15. Gulec S, Mesloras G, Dezarn W et al. Safety and efficacy of Y-90 microsphere treatment in patients with primary and metastatic liver cancer: tumor selectivity of the treatment as a function of tumor to liver flow ratio. J Transl Med. 2007;5–15.
Metadata
Title
Non-Functioning Pancreatic Neuroendocrine Tumors—A Case Report and Review of Literature
Authors
Murtuza M. Rampurwala
Anupam Kumar
Subramanian Kannan
Pamela Kowalczyk
Surendra Khera
Publication date
01-12-2011
Publisher
Springer US
Published in
Journal of Gastrointestinal Cancer / Issue 4/2011
Print ISSN: 1941-6628
Electronic ISSN: 1941-6636
DOI
https://doi.org/10.1007/s12029-010-9223-3

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